Autoimmune encephalitis and gastrointestinal dysmotility: achalasia, gastroparesis, and slow transit constipation

Abstract

Background Neurological autoimmune disorders (NAD) are caused by autoimmune inflammation triggered by specific antibody subtypes. NAD may disturb the gut-brain axis at several levels including brain, spinal cord, peripheral, or enteric nervous system.

Case report We present a case with antinuclear neuronal Hu (ANNA-1)- and antiglial nuclear (SOX-1) autoimmune antibody-positive limbic encephalitis and significant gastrointestinal dysmotility consisting of achalasia type II, gastroparesis, altered small intestinal interdigestive motility, and severe slow transit constipation. The autoantibodies of the patient’s serum labeled enteric neurons and interstitial cells of Cajal but no other cells in the gut wall. Achalasia was treated successfully by pneumatic cardia dilation and gastrointestinal dysmotility successfully with prucalopride.

Conclusion NAD may disturb gastrointestinal motility by altering various levels of the gut-brain axis.

Zusammenfassung

Hintergrund Neurologische autoimmune Erkrankungen (NAD) werden durch autoimmun -getriggerte spezifische Antikörper vermittelt, die die unterschiedlichen Ebenen der Darm-Hirn-Achse befallen können.

Fallbericht Wir berichten über einen Fall mit antinukleärer neuronaler Hu (ANNA-1) und anti-glialer nucleärer (SOX-1) autoantikörperpositiver limbischer Enzephalitis mit signifikanten gastrointestinalen Motilitätsstörungen mit Achalasie Typ II, Gastroparese, gestörter interdigestiver Dünndarmmotilität und schwerer Slow-Transit-Obstipation. Die Autoantikörper des Patientenserums markierten in der Darmwand nur Nervenzellen und Schrittmacherzellen. Die Achalasie konnte erfolgreich durch pneumatische Kardiadilatation und die gastrointestinalen Motilitätsstörungen konnten erfolgreich mit Prucaloprid behandelt werden.

Schlussfolgerung NAD können die gastrointestinale Motilität auf verschiedenen Ebenen der Magen-Darm-Achse schwächen.

Key words

autoimmune encephalitis - limbic encephalitis - achalasia - gastroparesis - slow transit constipation - autoimmune antibodies - ANNA-1 - anti-Hu

Schlüsselwörter

autoimmune Enzephalitis - limbische Enzephalitis - Achalasie - Gastroparese - Slow-Transit-Obstipation - Autoantikörper - ANNA-1, anti-Hu

Publication History

Article published online:
09 October 2020

• References

• 1 Malagelada JR. The brain-gut team. Dig Dis 2020; 38: 293-298
• 2 Blackburn KM, Kubiliun M, Harris S. et al. Neurological autoimmune disorders with prominent gastrointestinal manifestations: a review of presentation, evaluation, and treatment. Neurogastroenterol Motil 2019; 31: e13611
• 3 Budhram A, Leung A, Nicolle MW. et al. CMAJ 2019; 191: E529-E534
• 4 Kelley BP, Patel SC, Marin HL. et al. Autoimmune encephalitis: pathophysiology and imaging. Review of an overlooked diagnosis. Am J Neuroradiol 2017; 38: 1070-1078
• 5 Muñiz-Castrillo S, Vogrig A, Honnorat J. Associations between HLA and autoimmune neurological diseases with autoantibodies. Autoimmun Highlights 2020; 11: 2
• 6 Wandinger KP, Leypoldt F, Junker R. Autoantikörper-vermittelte Enzephalitiden. Dt Ärzteblatt 2018; 115: 666-673
• 7 Piepgras J, Höltje M, Michel K. et al. Anti-DPPX encephalitis: pathogenic effects of antibodies on gut and brain neurons. Neurology 2015; 85: 890-897
• 8 Kahrilas PJ, Bredenoord AJ, Fox M. et al. The Chicago Classification of esophageal motility disorders, v3.0. Neurogastroenterol Motil 2015; 27: 160-174
• 9 Wandinger K, Leypoldt F, Junker R. Autoantibody-mediated encephalitis differential diagnosis in patients with impaired consciousness of unclear origin. Dtsch Arztebl Int 2018; 115: 666-673
• 10 Li Q, Michel K, Annahazi A. et al. Anti-Hu antibodies activate enteric and sensory neurons. Sci Rep 2016; 6: 38216
• 11 Lütt A, Michel K, Krüger D. et al. High prevalence and functional effects of serum antineuronal antibodies in patients with gastrointestinal disorders. Neurogastroenterol Motil 2018; 30: e13292
• 12 Knowles CH, De Giorgio R, Kapur RP. et al. The London Classification of gastrointestinal neuromuscular pathology: report on behalf of the Gastro 2009 International Working Group. Gut 2010; 59: 882-887
• 13 De Giorgio R, Sarnelli G, Corinaldesi R. et al. Advances in our understanding of the pathology of chronic intestinal pseudo-obstruction. Gut 2004; 53: 1549-1552
• 14 Furuzawa-Carballeda J, Torres-Landa S, Valdovinos MA. et al. New insights into the pathophysiology of achalasia and implications for future treatment. World J Gastroenterol 2016; 22: 7892-7907
• 15 Furuzawa-Carballeda J, Aguilar-León D, Gamboa-Domínguez A. et al Achalasia – an autoimmune inflammatory disease: a cross-sectional study. J Immunol Res 2015; 2015: 729217 [PMID: 26078981 DOI:10.1155/2015/729217]
• 16 Kallel-Sellami M, Karoui S, Romdhane H. et al. Circulating antimyenteric autoantibodies in Tunisian patients with idiopathic achalasia. Dis Esophagus 2013; 26: 782-787